abstract

Cotard syndrome is usually described as an illusion that a person is dead or does not exist. Jules Cotard's original description of "negative illusion" (1880) is much richer, and it also involves the illusion and proposition of immortality and greatness, the feeling of curse, and the illusion of body dissolution and transformation. Or extreme cases of depression, hypochondriasis or mental illness, are considered rare and people know little about it. Cotard himself provided a classification and several explanations for this situation, focusing on the difference between it and the classic persecution delusion, and pointed out that this may be an inverted grandeur. He put forward the spiritual feeling basis of mental image decomposition, and then extended it to the more common mental movement disorder of will. Other early authors emphasized the disorder of body self, while the recent theory assumed that the right hemisphere function was impaired, which led to the unreality of perception and body feeling, coupled with reasoning obstacles and internalized attribution methods, which led to the non-existence of beliefs. However, although Cotal's syndrome is remarkable and related to our understanding of self-consciousness, it is still an elusive disease, which is rarely reported and studied.

introduce

The beliefs and experiences reported by patients with Cotal's syndrome directly contradict Descartes' famous creed. Descartes' famous creed is that one thing can never be doubted, and that is our own existence, which is embodied in the sentence "I think, therefore I am". In fact, these patients often claim that they are dead or no longer exist, so they may show "the only self-confirmed mental delusion in all medicine" [1] and a specific reduction to absurdity (in this case, it looks like "I think, so I am not here" [2]).

However, this is based on the French psychiatrist Jules Cotal (1840-1889; For details of the biography, see [1, 3]). The special syndrome named after it involves more complicated pictures, rather than the short description of "the illusion of a person's death" that is often quoted. Although it raises profound philosophical questions and provides insight into the potential neurocognitive mechanism, our sense of existence, the formation of faith and the limitations of personal knowledge and consciousness are still a medical mystery to a great extent.

For at least two years, Mlle X… has been worried about "internal cracks from the back to the head", and then a series of complicated symptoms appeared: a devout woman, who thought that she had been cursed by God all her life, claimed that she had "all kinds of self-blaming mistakes" and reported that she had "no brain, no nerves, no chest, no stomach and no internal organs", and she only had messy body and skin. Kota wrote that this "illusion of negation" extends to "metaphysical thought:" She has no soul, God does not exist, and the devil does not exist. Therefore, she "can live without eating, and will not let her die naturally, unless she is burned to death, she will exist forever." In fact, she not only begged to be burned to death, but even tried to do so. Extreme excitement and violence can also infect others, and she will bite, scratch and hit them. Clinically, Cotard reported bilateral insensitivity to pain ("a person can deeply press the needle (in her body) without showing any pain sensation"), while other body sensory functions are completely preserved.

Cotard quickly put forward similar old literature cases, especially five cases of "devil's disease" from "gentleman's disease" (1838): these patients claimed that they had no body and blood, they were cursed, they would live forever, one was a "body statue", and the other felt "empty". "Some people thought their bodies were rotten. Cotard also distinguishes this obstacle from persecution paranoia, on the grounds that pure paranoid patients tend to put forward the idea of being attacked again, and rarely mention metaphysical negation, although in this new clinical picture, the statement of self-destruction is repetitive and seems to be final and all-encompassing (in Ceglarz's words: "There is an abyss between the long speech of the depressed person and the novel of the persecuted person" [1/kl.

Although contradictory at first glance, Cotard thinks that some of his patients infer from their state that they must be immortal, which may have "certain logic". In fact, although immortality seems to contradict the belief in death, Cotal pointed out that these patients think that their bodies are not in a normal state that allows death. "If they could die, they would have died", and they are "in a state of neither birth nor death": in short, "they are alive and dead", contrary to the grand fantasy of "long-persecuted megalomaniacs", they hope to learn from them.

Cotard suggested that this new clinical manifestation should be summarized as a state similar to "anxiety and depression", but the element of "paranoia" was added to make this situation chronic. He finally put forward a set of six characteristics that define this obstacle, "if it should be isolated." They are: (1) depression and anxiety; (2) the concept of curse or possession; (3) the tendency of suicide and self-mutilation; (4) analgesia; (5) hypochondriacs-all kinds of organs do not exist or are damaged, the whole body, soul, spirit, etc.; (6) the concept of immortality.

It can be said that the first report contains all the main features of Cotard syndrome, which will be discussed in the next few years. However, two years later, Cotard published follow-up papers, including 1 1 new cases and more elaborate theoretical methods [12]. This disease is now officially named "délire des négations" (negative hallucinations), which refers to the tendency of these patients to deny, reject and oppose everything that is said or provided to them. Other features now include: denying the existence of others, objects and the outside world; Visual and auditory hallucinations; Repeated self-deprecating words or mumbling; Talk about yourself in the third person; Feeling of body displacement and deformation (for example, as an animal); Misrecognition of others; The idea of possession and the delusion of external influence; Silence and immobility. Divided into three categories: simple denial of delusion (8 cases); Denial of delusion is a symptom of general paralysis of mental patients (1 case); Negative delusion is related to persecution delusion or appears alternately (2 cases). The latter kind of "mixing" is considered to be an intermediate state between guilt delusion and persecution delusion, which leads people to believe that they are possessed by demons or animals. In addition, Kotar associated immortal beliefs with very anxious situations, and death beliefs with more coma and depression situations, allowing mixed or alternating States.

In the final clinical supplement, Cotal emphasized an interesting similarity in 1888, that is, his illness and his great delusion. He pointed out that patients with anxiety, depression and hypochondriac paranoia often use exaggerated images in their chief complaints: they not only declare eternal life, extreme self-disintegration and the end of the world, but also report the feeling of "greatness": "Some of them [immortals] are infinite not only in time, but also in space. They are huge, huge, and their heads will touch the stars (…). Sometimes the body has no limit, stretches infinitely and blends into the universe. These useless patients have become everything. " However, "in their exaggeration and greatness, their thoughts retain their terrible side." He thinks this is a kind of "pseudo-arrogance" or "the opposite of the great illusion". Although Kotar died suddenly in 1889, leaving others to continue to discuss his syndrome, in the following years, the overall clinical picture did not increase significantly (on the contrary, the scope of this syndrome became smaller and smaller, which helped to maintain its unique, mysterious and eye-catching aura.

epidemiology

Although Ceglarz thinks that negative opinions are quite common in depression and other diseases, he thinks that Cotal's syndrome is "quite rare" because he was not only named after Cotal at the suggestion of Emile Regis in 1893, but also made great contributions to popularizing this disease. "And this idea is still prevalent. Although Cotard's suggestion of a new clinical entity immediately aroused people's interest, theoretical discussions and new case reports were generated in the following years [3, 10, 14,15]; It is difficult to compare the scattered reports of Cotard syndrome systematically, and it is also difficult to estimate its incidence or prevalence reliably.

However, berrios and Luke [16] analyzed 100 cases published since 1880. Although their method was specially put forward for the author's purpose (reviewing the evolution of clinical concepts since their birth), of course, this method is biased because it only reviews cases that are considered worthy of publication, but it has produced some interesting observations. Their sample age range is 16-8 1 year, with an average of 52 years old. The only difference between male and female patients is that the latter is more prone to organic diseases. Age seems to indicate the occurrence of nihilistic delusions about the body and self-existence. Physical delusion is the most common nihilistic delusion, accounting for 86% of the reported cases, followed by denial of existence (69%), and the incidence of depression is 89%. In a recent analysis, similar results were obtained by adding 38 cases, but this time it showed that the occurrence rate of women in case reports was higher in history (68%; [17]), which provides some evidence for the impression of Enoch and Tretow Wan, that is, gender factors are related to Cotard syndrome (that is, more women than men [18]); See also [19]).

A two-year study in a three-level referral center in Mexico found that among the patients with neurological and mental diseases in 132 1 (0.3%), four patients suffered from Cotal's syndrome. Among 842 patients with nervous system diseases, 1 patient (0. 1%) developed Cotal syndrome (secondary to non-herpetic viral encephalitis), and among 479 patients (0.62%) with pure psychosis, 3 patients (20%) developed Cotal syndrome. Chiu(2 1) found that in the study of 0.57% years, there were 0.57% (349 out of 349 cases were Cottle syndrome). These two patients were 67-year-old and 70-year-old female patients with major depression (18. 1% of all patients with major depression, and the prevalence rate of Cotard syndrome was 3.2%). These two studies not only emphasize the rarity and difficulty of diagnosis of the syndrome, but also point out the cross-cultural similarity of the disease. Soultanian et al [19] It is estimated that the annual prevalence of Cotard syndrome in adolescents and young people is lower than11000. They reported 4 cases (all female) of children and adolescents' psychiatry in 10, and found 9 cases of1Cotard syndrome published in French and British literature before 2003. Whether the prevalence and characteristics of Cotal's syndrome are stable in history or have changed over the years is an open question, but the content of delusion must change with the change of culture [22].

pathogenesis

It is difficult for Cotal's syndrome patients to have a formal test in acute delusion: they may be excited, desperate, inattentive or object to any problem. The case report of a semi-illiterate patient in Leafhead and Koppelman [23] proves this difficulty, and the patient needs four instability tests. Therefore, most information about this syndrome is purely observational. Despite the lack of empirical research, or perhaps because of this, there are still many speculations about the etiology and pathogenesis of Cotard syndrome.

Cotal himself has a different understanding of his syndrome. Anxiety, depression and depression (depression) lead to specific fantasies about one's own destiny, present situation and existence, which sometimes extend to the outside world, which is his starting point [7, 12]. He tried to distinguish these patients from the more typical persecution paranoia, emphasizing their unique physical experience, deep despair and internalized interpretation style, and let them infer their concrete and lasting feelings about death and/or immortality according to death and/or immortality, sin (curse) and loss of identity (possession and greatness). In 1884, he reported two new cases and was inspired by a recent paper by his tutor Jean-Martin Charcot. He proposed a possible mechanism to explain the emptiness that troubled these patients, namely "Perte de la vision mentale" (24). It is reported that in both cases, they are unable to "mentally reproduce what they are most familiar with", including the urban terrain, the faces of family members and personal documents. Cotal believes that the coexistence of nihilism and imagination disorder may be "not just accidental coincidence". If this is the case, then people will "imagine systematic negation as a delusion grafted on psychological sensory disorders, a pathological explanation of this phenomenon." He also pointed out that it is difficult to evaluate the psychological representation of patients who deny the existence of things. However, in his last article [25-27] before he died of diphtheria at the age of 49, Cotal gradually gave up this "psychological-feeling" method and developed a pure "psychological-movement" illusion theory. Regarding the delusion of negation, he envisaged a mechanism, that is, the potential "depression of mental movement energy" (modern term, the damage of movement consciousness or agency consciousness) leads to the loss of contact with spiritual images and the outside world, which in turn leads to emptiness and unreal anxiety. In the worst case, patients will eventually explain these emotions in nihilistic terms [27].

Therefore, Kotar's view of his own syndrome contains many explanations: serious emotional disorder (anxiety and depression), physical misunderstanding and hypochondriasis, persecution delusion manifested as curse and/or possession, loss of mental image, impaired motor consciousness and will, and defective reasoning. Although these views lack empirical support, people can only be impressed by their sensibility and modernity. For example, Séglas[ 1 1] soon put forward the concept of damage to the center of body consciousness. He believes that the central obstacle of the body self explains strangeness and disappearance, denial and lack of ownership, as well as the changes in sensory processing of body parts and the whole body, or cenestopathie. Jean Lhermitte[28] further developed this method, and he coined the term "total insensitivity" as an analogy of unilateral neurological disorder in body mode (see also [29, 276-282] and [30]). The loss of mental image is now studied in the name of "aphasia", which seems to be a real entity, although people with this disease are not delusional and have not been formally studied in patients with Cotard syndrome [3 1]. Cotal's works also preceded the recent discussion on "concretization" and "motor cognition", the relationship between motor cognition and its role in psychopathology (for example, [32]). Similarly, Kotar and Ceglarz clearly foresee the emergence of modern delusional thinking theory, which is based on the cognitive elaboration and misinterpretation of unusual or nonexistent feelings. According to the observation that Cotard syndrome and Capgras syndrome (or more generally, misrecognition syndrome) may occur in the same patient at the same time (simultaneously or successively), and the discovery that the performance of patients with Cotard syndrome is impaired in facial memory (and/or processing) tasks, Young and leadhead[2] assume that Cotard syndrome is caused by facial memory (and/or processing) defects, unreality (. Therefore, patients in Cotal lose their familiarity with others and themselves, feel strange and illusory in their hearts, and blame themselves for the development of the situation, thus concluding that they must be worthless, rot internally, die or do not exist (while capgras and persecuted patients tend to blame their situation on external factors). However, it is not clear how this inference came about: in 1905, Deny and Camus[34] reported a patient with anxiety and depression. She claimed that she couldn't perceive her body, and everything was untrue. She lost the ability to form mental images. She felt dead and everyone around her was like a ghost. She had to keep touching herself to convince herself of her existence, but she didn't. In this case, it is closely related to serious personality disintegration, which requires an additional obstacle to "completely deny a person's material existence" (see also [28]); For a modern discussion of this two-factor delusion theory, see [35]).

Cohen and Consoli[22] pointed out that the delusional content of Cotard syndrome is related to personal biography and culture. For example, although elderly patients with Cotal's syndrome show obvious fear or paranoia, involving syphilis infection, AIDS has been cited in recent cases. Curse and possession may be out of date now, so it will be interesting to track the changes of nihilistic delusions and relate them to personal history (in modern cases). Neuroanatomy, neurofunctionalism and neuropsychology of Cotard syndrome have produced opposite results. Kudlur et al. 36 (see also 37) reviewed all 37 related case studies before 2007 and found that the right hemisphere and frontal lobe were often involved, but not the only ones. There are reports of multifocal brain atrophy, hemispheric fissure enlargement and ventricular enlargement, as well as focal lesions in frontotemporal bone or temporal parietal region. A recent case report highlighted the atrophy of bilateral insular cortex in a woman with Cotard syndrome of depression and insanity, which indicated that her interosseous sensation and body consciousness were impaired [38]. There are various causes of nervous system, including tertiary syphilis infection, stroke, tumor, temporal lobe epilepsy, migraine, mental retardation, traumatic brain injury, Parkinson's disease, arteriovenous malformation and multiple sclerosis. Except for patients with epilepsy, the clinical EEG records of patients with Cotard syndrome are usually not obvious. The recent FDG-PET study of a single patient revealed a broad cortical hypometabolism network, involving the frontal parietal lobe and midline structures, such as anterior cingulate gyrus, anterior cingulate gyrus and posterior cingulate gyrus, as well as the key structures of the "default network" of potential self-processing and core consciousness functions [39].

diagnose

For the detailed description of Cotal and his followers, and the heated discussion after his new clinical photos, especially after his death, it seems that there is no recognized standard and official diagnosis to formally determine whether a specific patient has Cotal's syndrome, or even to decide whether it represents all symptoms, a syndrome, a symptom or a series of symptoms, an extreme depression, a body pattern or physical disorder, or paranoia, hypochondriasis and (. To illustrate these difficulties, let's take a look at the case report of "Mrs. Zero" by capra Sey and Damson. This woman has the idea of combining negativity with mania. She repeatedly sings the sentence "When a person doesn't exist, hell is here", which seems to make fun of the doctor. She was labeled as "atypical Cotal syndrome".

Jules Ceglarz [1 1] distinguishes between negative delusions (Cotal syndrome itself, which belongs to severe anxiety and depression) and negative thoughts (hypochondria, bipolar disorder, insanity, brain injury, alcoholism, mental retardation, etc. In Ceglarz's view, Cotal syndrome is the existence of (1) negative ideas (related to a person's personality and cognition, identity, body, body parts and body functions), and the external world consists of objects, nature and people, abstract ideas, such as God or existence-"nothing these patients can't deny"-and the idealization or "retrospective illusion" of the past self. (2) the concept of immortality and greatness ("pseudo-arrogance" and "exaggeration"); (3) Melancholy delusion with curse or possession concept (self-mockery, self-reproach, "humble delusion", fixed monotonous complaint, morbid expectation); (4) Emotional and hallucinatory disorders (anodynia or hyperalgesia, sensory distortion, psychomotor and proprioception changes, body deformation and displacement, thirst, hunger and visceral loss, multimodal hallucinations; (5) Anxiety reaction (excitement, opposition, silence, verbal persistence, refusal to eat, self-mutilation, suicide). However, as Cotard himself described (see above), it is not clear whether all or any characteristics are necessary for Cotard syndrome.

Young and Leafhead[2] analyzed the clinical features of 8 "pure cases" reported by Cotard in his paper 1882 (namely "simply denying delusion"). They listed 3 1 symptoms, from which six categories were extracted: general nihilism (denying others or the environment); Self-delusion (denying yourself and your body and believing that you are dead and not dead); Self-mockery and delusion (remorse, guilt, imaginary fear, curse); Physical hallucinations (hypochondria, denial, corruption, stench, changes and deformation of body parts); Abnormal perception (multimodal hallucination, hallucination, anesthesia and hypersensitivity); Behavioral manifestations (suicidal attempts and thoughts, violence, self-hunger, silence, immobility, etc.). ). All cases have no single symptom, only 1 death 1 case. In fact, the most common aspects are self-mockery delusion, behavior and body delusion, which exceeds the existence of nihilistic delusion (autocorrelation or general). Therefore, the author warns against using the word "syndrome" without introspection, especially when it comes to Cotal's illusion that a person should mainly die (however, in this chapter, we use the word "syndrome" quite loosely because it is adopted at the end of 19). Berrios and Luke [14, 16] also regret the tendency to regard Cotal syndrome as an isolated illusion of a person's death. Using Cotard index established for practical purposes to evaluate 100 published cases (it is only the total number of reported nihilistic delusions about "body, existence or concept"), they conducted exploratory factor analysis and got three types: patients with mental depression (depression with a small number of nihilistic delusions); Co tard type ⅰ patients (patients with mild depression but high degree of nihilism and delusion, namely "pure Cotard syndrome", are closer to delusion in pathology than emotional disorders); And Cotard II cases (anxiety, depression and auditory hallucination mixed group) [16].

These observations provide useful insights for the disease, and put forward methods to quantify or classify cases that suggest Cotal's syndrome, but there are no clear or official standards and tools at present. Unfortunately, as far as our overall understanding of this situation is concerned, it partly explains the lack of reliable epidemiological data, the diversity of etiological pathways, the differences in neurobiological findings and some approximate methods for diagnosis and differential diagnosis. Cotard is most interested in the differential diagnosis between persecution delusion (he provided a complete table in [12]), hypochondria, depression and delusions. It is not clear whether Cotard syndrome should be distinguished from underlying or accompanying pathologies, such as schizophrenia, brain diseases, emotional disorders, separation status, body pattern disorders and misidentification syndrome. For example, recently, Soultanian et al. [19] Consorti et al. [17] suggested that the early onset of Cotard syndrome (adolescence or adolescence) is closely related to the subsequent development of bipolar disorder, and suggested that these patients should be closely monitored, including using ECT and mood stabilizers when necessary. These findings raise some interesting but unsolved problems, such as the onset and course of Cotard syndrome and its relationship with other diseases (and the difference from other diseases).

treat cordially

The concept of Cotal's syndrome and the difficulty of diagnosis directly reflect the problem of how patients should be treated. From the beginning, it can be said that by definition, this situation is considered serious. These patients are not only isolated, depressed and extremely uneasy, but also in serious danger of self-harm. Death due to self-starvation, illness or suicide is sometimes reported as a result (for example, [4 1-43]). However, attacks on others are even rarer, and only two patients have reported them, including cases of princeps [7 7,44]. It is suggested to intervene in monitoring as soon as possible. Moral problems may arise because these patients may sometimes object and refuse help or treatment.

Although Cotard initially thought this situation was "chronic", many cases of complete (sometimes spontaneous and sudden) recovery have been reported, usually due to the elimination of potential confusion or depression. Therefore, Enoch and Ball[ 18] suggested that the treatment of Cotard syndrome should depend on the potential diagnosis, which seems reasonable, at least until more people know the exact nature (or reality) of the disease. Therefore, before considering treatment, we should carefully evaluate the emotional, mental and organ aspects of the speech. Literature [37] provides a detailed list of successful drug interventions in reported cases, including fluoxetine, paroxetine and lithium, as well as the combination therapy of haloperidol and clomipramine. Patients with depression or psychotic depression may be the easiest to accept ECT, which seems to be the most frequently reported successful treatment, especially after drug maintenance therapy combined with ECT. Berrios and Luke [16] think that patients with high degree of delusion but low degree of emotional disorder ("simple Cotard syndrome" or "co tard I type") should have lower response to antidepressant treatment. It remains to be seen whether they are the easiest to receive antipsychotic drugs.

The neurocognitive theory of Cotard syndrome puts forward another more accurate method. If this situation is really caused by different combinations of strangeness, depersonalization and loss of reality, and depression involving internal attribution, cognitive and neuropsychological rehabilitation therapy may try to enhance and recalibrate familiarity with others, the outside world and one's own body, and/or restore a more realistic and neutral causal explanation. However, this may require a special training plan according to the specific situation.

Conclusion and future direction

Cotal's syndrome, as a special, rare and unsolved disease, naturally aroused people's theories and speculations. Although most questions surrounding this situation are still completely open, few people doubt that Jules Cotard's initial report is charming and prescient. His syndrome uniquely contains medical, psychological, philosophical and existential problems, which may explain his long-term confusion in essence. Ironically, this great contribution may now be in danger. Due to the progress of biomedical research and neuroscience, in the crisis of psychopathology classification, in an era like radio rumors and sensational trivia, Cotal's syndrome is often simplified as "a person believes that he is dead". Although this is attractive and easy to be dramatized in popular culture, Cotal's unique heritage, which goes deep into human conscience and suffering, may be greatly damaged. It is more appropriate to remember and carry forward his past contributions, because he is the source of inspiration for Marcel Proust, a meaningful memory master.

There is still a lot of work to be done before the eulogy of Cotal's syndrome is published and the autopsy is performed. Of course, more neuroimaging research will promote their understanding, but psychological and clinical research and observation will be the center if we want to make progress. It is worth noting that Cotal and his followers put forward their views on the treatment of sensation and pain, the feeling of body ownership and initiative, the function of psychological images, the expression of speech acts, the components of psychological ontology, the evaluation of human death consciousness, and the relationship between emotion, perception and motor system more than a century ago, which are now in the key aspects of modern technology's Cotal syndrome.